Access the full text.
Sign up today, get DeepDyve free for 14 days.
References for this paper are not available at this time. We will be adding them shortly, thank you for your patience.
Hindawi International Journal of Surgical Oncology Volume 2020, Article ID 9197216, 7 pages https://doi.org/10.1155/2020/9197216 Research Article The Management of Desmoid Tumors: A Retrospective Study of 30 Cases 1 1 2 1 1 Yosr Zenzri , Yosra Yahyaoui, Lamia Charﬁ, Zahra Ghodhbani, Feryel Letaief , 1 1 Mouna Ayadi, and Amel Mezlini Medical Oncology Department, Salah Azaiez Institute, Tunis, Tunisia Pathology Department, Salah Azaiez Institute, Tunis, Tunisia Correspondence should be addressed to Yosr Zenzri; email@example.com Received 16 April 2020; Accepted 1 July 2020; Published 18 July 2020 Academic Editor: C. H. Yip Copyright © 2020 Yosr Zenzri et al. (is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Objectives. Desmoid tumor also called aggressive ﬁbromatosis is a rare type of benign tumor. It is a mesenchymal malignancy without metastatic potential. (e standard management is resection, but other options including observation may be discussed. Desmoid-type ﬁbromatosis may occur throughout the body, but the abdominal wall is the most common site. (e aim of our study was to assess the clinicoepidemiological proﬁle, prognostic factors, and treatment outcome of desmoid tumors. Methods. A monocentric retrospective study was conducted over a period of 19 years between February 2000 and November 2019 at the oncology department of Salah Azaız Institute. Our study concerns 30 patients with desmoid tumor. All data regarding patients were obtained from the medical record. Results. (irty patients were included. (e median age was 35 years with a female predominance (sex ratio � 0.07). A palpable mass was the most common complaint (n � 27). Median tumor size was 5 cm. (e principal site of involvement was the abdominal wall (n � 14). Surgery was performed in 27 patients. (e histopathology reports listed 14 (52%) cases with negative margins and 13 (48%) cases with positive margins. Radiation therapy was performed in 2 patients. One patient received tamoxifen. Local recurrence occurred in 11 patients. Two patients died of their desmoid tumor. Abdominal wall tumors have less risk of recurrence compared with other sites (p � 0.047). Macroscopic margin involvement (R2) was the only prognostic factor inﬂuencing disease-free-survival (p � 0.034). Conclusion. Desmoid tumors are aggressive tumors with a tendency for local recurrence. Abdominal wall tumors have less risk of recurrence. Macroscopic margin involvement was the only prognostic factor that aﬀects disease- free-survival. (e clinical behavior and natural history of aggressive 1. Introduction ﬁbromatosis remain unpredictable. Aggressive ﬁbromatosis also called desmoid tumors (DTs) (e symptoms depend on location and size of the tumor. are soft tissue malignancies originating from fascial planes, (e presentation can vary from asymptomatic to severe connective tissues, and musculoaponeurotic structures of pain, swelling, deformity, and loss of function. the muscles . DTs are rare and constitute less than 3% of In fact, these tumors can be very aggressive with rapid all soft tissue tumors and 0.03% of all neoplasms . (ey growth and mass eﬀect while others can be indolent and are typically diagnosed in young adults with a female pre- spontaneously regress . dominance . (e exact etiology of these tumors is unknown but DTs are a benign proliferation of myoﬁbroblasts. (is trauma, hormonal, and genetic factors are considered to be entity does not have the ability to metastasize. Although correlated with the development of DTs . benign, these tumors can be locally invasive, causing pain or Wide surgical excision remains the mainstay for re- deformity that could make surgical removal more diﬃcult. sectable aggressive ﬁbromatosis. But, alternative treatment 2 International Journal of Surgical Oncology modalities including radiation therapy, chemotherapy, ta- history of a surgical procedure at the site of the tumor, while moxifen, tyrosine kinase inhibitors, and nonsteroidal anti- 2 patients recalled nonsurgical trauma at the site of tumor. inﬂammatory drugs may be used as a treatment for recurrent None of the patients had history of familial adenomatous disease or as primary therapy to avoid mutilating surgical polyposis (FAP). resection . (e diagnosis of aggressive ﬁbromatosis was made (e aim of our study was to assess the clinicoepidemio- during the ﬁrst trimester of pregnancy in one patient. A logical proﬁle, prognostic factors and treatment outcome of palpable mass was the most common complaint (n � 27). Six DTs. patients were complaining of extreme pain. One patient present with a swelling and functional disability. DTs were located in the extremities (n � 9; 30%), abdominal cavity 2. Materials and Methods (n � 4; 13%), abdominal wall (n � 14; 47%), or thorax (n � 3; 2.1. Study Design. A retrospective study including all con- 10%). (e median size of the tumor was 5 cm (range, secutive patients with DTs was performed at a tertiary-level 2.5–17.5 cm). hospital (Salah Aza¨ız Institute) from February 2000 to Eighteen patients (60%) received magnetic resonance November 2019. (is study has been approved by the in- imaging (MRI) examination, and nineteen (63%) had ul- stitution’s Ethics Committee. Consent to participate was trasonography. (e diagnosis was based on histological waivered do to the retrospective nature of the study. Data samples obtained by core-needle biopsy in 20 patients were collected and handled anonymously. (e diagnosis of (67%).Characteristics of the 30 patients included in our DTs was based on clinical, radiological, and histological study are detailed in https://www.ncbi.nlm.nih.gov/pmc/ criteria. Only patients with histologically conﬁrmed ag- articles/PMC3568525/table/T1/ Table 1. gressive ﬁbromatosis who received their treatment at a study Wide surgical resection was performed in 27 patients center were included. (e histological diagnosis was made (90%). In 14 cases, a wide (R0) resection, in 11, a marginal by the pathologists of the institute. (R1) resection, and in 2 cases, an intralesional (R2) re- section was achieved. Immunohistochemical analysis revealed that the tumor cells were strongly positive for 2.2. Data Collection. Data extracted as part of this retro- beta-catenin in 10 patients (33%). Figures 1 and 2 illustrate spective analysis included age at diagnosis, gender, date of the macroscopic and microscopic aspects of the desmoid diagnosis, tumor size, primary site, surgical margins, re- tumors. currence rate, date of progression, time to progression, date Two cases were managed by simple observation. Two of last follow-up, and survival status. patients with R1 resection underwent adjuvant radiation Lesions at the buttock, axilla, and groin were classiﬁed as therapy. One patient with R2 resection received tamoxifen extremity. Lesions at the neck, because of their small number after surgery. in our study (n � 1), were considered with extremity lesions. One patient developed an acute respiratory failure and Margin was classiﬁed as gross positive (R2), microscopic died before starting any treatment. positive (R1), or negative (R0). Treatment characteristics are detailed in Table 2 (https:// Recurrence was deﬁned as a lesion that histological www.ncbi.nlm.nih.gov/pmc/articles/PMC3568525/table/T1/). examination has proven recurrent aggressive ﬁbromatosis or (e median follow-up was 40 months. Local recurrence a lesion that was deemed suspicious on imaging. (LR) occurred in 11 (38%) patients within a median time interval of 21 months (range, 1–60 months). Of the 11 patients with LR, 6 had positive margins after initial surgery. 2.3. Statistical Analysis. (e data were analyzed on the Eight patients (73%) underwent repeat resection, 2 patients Statistical Package for the Social Sciences (SPSS) version 20 were lost to follow-up, and one patient developed bowel (IBM SPSS, Armonk, NY, USA). Results were expressed as obstruction and died before resection. Two patients had mean± standard deviation. Univariate comparisons be- adjuvant radiation therapy. Four of these 8 patients had a tween groups were performed using chi-squared tests for second relapse. An attempt at another resection was un- dichotomous variables or Fisher’s test when appropriate. For dertaken in three patients. One patient was lost to follow-up. continuous variables, independent samples t tests were used. One of these three patients developed a subsequent recur- P< 0.05 was considered to be signiﬁcant. rence during follow-up. Cumulative event rates were calculated using the method (e 1 and 3-year recurrence-free survival was 27 and of Kaplan–Meier. Survival curves were compared using the 18%, respectively (Figure 3). log-rank test. Recurrence-free survival was determined as Abdominal wall tumors had less risk of recurrence the time from diagnosis to either histology-proven or ra- compared with other sites (p � 0.047). diologic evidence of disease recurrence. Macroscopic margin involvement (R2) was the only prognostic factor inﬂuencing disease-free-survival 3. Results (p � 0.034) (Figure 4). (e median age of the 30 patients at study inclusion was 35 In univariate analysis, age, history of trauma, and size of years (range, 18–80 years). Patients with aggressive ﬁbro- the tumor did not show a signiﬁcant impact on local re- currence. (e multivariate analysis could not be performed matosis were predominantly female (n � 28; 93%). (e av- erage delay in consultation was 6 months. (ree patients had due to the limited number of patients. International Journal of Surgical Oncology 3 Table 1: Characteristics of patients. Treatment Frequency % Sex Male 2 7 Female 28 93 Median Age � 35 years History of a surgical procedure at the site of the tumor 3 10 History of nonsurgical trauma at the site of tumor 2 7 History of pregnancy in females with tumor 1 3 Complaints Palpable mass 27 90 Pain 6 20 Swelling and functional disability 1 3 Location of primary tumor Abdominal wall 14 47 Extremities 9 30 Abdominal cavity 4 13 (orax 3 10 Median size of the tumor � 5 cm Imaging ultrasonography 19 63 MRI 18 60 CT scan 12 40 Biopsy 20 67 (a) (b) Figure 1: Macroscopically, the tumors were tenacious, yellowish white on the cut. (a) (b) Figure 2: Interlacing bundles of ﬁbroblasts separated by variable amounts of collagen in extra-abdominal ﬁbromatosis. Regularly dis- tributed blood vessels are conspicuous. 4 International Journal of Surgical Oncology Table 2: Treatment characteristics. 4. Discussion Treatment Frequency % Aggressive ﬁbromatosis is uncommon and accounts for Wide surgical resection 27 90 only 0.03% of neoplasms and less than 3% of soft tissue Surgical margins tumors .(ey are described as a clonal ﬁbroblastic R0 14 52 proliferation in soft tissues with inﬁltrative growth . R1 11 41 (ey are locally aggressive tumors with a high rate of local R2 2 7 recurrence after surgery but rarely metastasize. Indeed, Beta-catenin 10 33 Observation 2 7 they have unpredictable clinical course. DTs can appear in Adjuvant radiation therapy 2 7 any anatomic location, frequently in the abdominal region: Tamoxifen 1 3 in our study, the tumor was located in the abdominal wall (47%), abdominal cavity (13%), extremities (30%), and thorax (10%). Other studies suggested a completely dif- ferent distribution: abdominal wall (17.4%), abdominal cavity (10. 8%), and extra-abdominal (69.5%) . (e 1.0 presentation can vary from asymptomatic to pain, defor- mity, swelling, or loss of function. (e exact causes of DTs 0.8 are largely unknown, but several factors may be involved. DTs usually occur in individuals between 15 and 60 years with a female preponderance : 93% (n � 28) in our study 0.6 and 67% in other series . But, there is an equal inci- dence in males and females in DTs associated with FAP 0.4 . Pregnancy is associated with DTs due to high es- trogenic levels . DTs are related to trauma mainly 0.2 surgical ones: 16% of our patients had a history of trauma. It seems to be one of the most important etiologic factors. Indeed, this can be explained by molecular connection 0.0 between ﬁbroproliferative disorders of mesenchymal tissue .00 10.00 20.00 30.00 40.00 50.00 60.00 and wound healing processes . Recurrence-free survival (months) DTs are mainly sporadic, but they can also be associated Figure 3: Recurrence-free survival analyzed by the Kaplan–Meier with familial inﬁltrative ﬁbromatosis, hereditary desmoid method. disease, Gardner syndrome, and familial adenomatous polyposis .10 to 20% of DTs are associated with FAP while 10–30% of patients with FAP develop desmoid tumors [14, 15]. Colonoscopy should be done for patients with DTs especially if they are young or have intra-abdominal or 1.0 abdominal wall tumor. Histological examination of DTs revealed some char- acteristics: paucicellular proliferation of ﬁbroblasts/myoﬁ- 0.8 broblasts in a dense collagenous background, spindle cells with small and regular nuclei, pale eosinophilic cytoplasm, and acellular central area with increasing cellularity to the 0.6 periphery . (e β-catenin positivity is caused by CTNNB1 mutation in chromosome 3p22. It is mainly found in sporadic DTs . In contrast, DTs associated with FAP 0.4 are caused by germline APC mutation in chromosome 5 (5q21e22) and then a somatic inactivation . Nuclear 0.2 positivity of β-catenin supports the diagnosis of DTs. Moreover, some studies suggested that a very strong β-catenin positivity is correlated with a high risk of recur- 0.0 rence . But, nuclear β-catenin is not always speciﬁc, it can be positive in palmer/plantar ﬁbromatosis, solitary ﬁ- .00 10.00 20.00 30.00 40.00 50.00 60.00 brous tumor, Gardner ﬁbroma, synovial sarcoma, and low- Recurrence-free survival (months) grade ﬁbromyxoid sarcoma and not all DTs will stain for Margins nuclear β-catenin: cytoplasmic staining is more sensitive but R2 less speciﬁc . R0 ou R1 MRI is the modality of choice for assessment of the nature and size of DTs. 60% of our patients has undergone Figure 4: (e Kaplan–Meier plot of local recurrence according to this imaging. DTs show some features in MRI: they have margin. Recurrence-free percentage Recurrence-free percentage International Journal of Surgical Oncology 5 Antihormonal therapies such as tamoxifen can be used stellar shape; they extend into the fascial planes and fat tissue in a sun-burst-like form. Besides, they are isointense on T1 alone or in association with NSAIDs as ﬁrst-line treatment. (ere are some suggesting that higher doses (up to 120 mg/ and hyperintense on T2 . In most series, local recurrence rates after surgical re- day) in combination with NSAIDs are more eﬀective than section depend on margins. Indeed, these rates range from tamoxifen alone . 80% to 10% when surgical margins are positive or negative, COX-2 seems to play a role in the pathogenesis of DTs. respectively . In our study, 11 patients (38%) had local NSAIDs has inﬂuence on the β-catenin pathway. NSAIDs recurrence: 6 patients had PM and 5 patients had NM. that inhibit COX may be an alternative treatment. Indo- Macroscopic margin involvement (R2) was the only methacin and sulindac were used in the treatment of DTs. As a result, partial and complete response was achieved in some prognosis factor inﬂuencing the disease-free-survival (p � 0.034). nonrandomized retrospective studies . But, the risk of cardiovascular events may be increased in patients receiving (erefore, positive margins seem to be the most im- portant indicator for an adjuvant treatment as radiation NSAIDs. (is treatment should be avoided for some fragile patients. therapy. Many studies showed the importance of radiotherapy. In (e chemotherapeutic option can be the treatment of a study published by Jelinek et al., adjuvant radiotherapy was choice in cases of unresectable, aggressively growing and/or the only signiﬁcant prognostic factor for local control. (e 5- life-threatening tumors. Many chemotherapy protocols have year local control rate was 53% for patients who underwent been used. In the pediatric population, weekly adminis- surgery alone and 81% for patients who had surgery and tration of methotrexate and vinblastine has been evaluated. radiation therapy (p � 0.018) . Skapek et al. found that this regimen was well tolerated, and the response rate was 19% in a cohort of 28 patients . (e eﬀects of involved surgical margins after surgery remain unclear. PM were predictors for failure in some But, there is no prospective data for this combination in adults. Antracyclines appeared to have a higher response studies  but not in others . In our study, abdomen wall tumors had less risk of rate. (is regimen is administrated for 6 to 8 cycles . Pegylated liposomal doxorubicin at a dose of 50 mg/m recurrence compared with that in other sites (p � 0.047). every (ere was no recurrence in a series of 7 abdominal wall 4 weeks showed acceptable toxicity and a signiﬁcant activity tumors published by Sutton and (omas . Shao et al. . Tyrosine kinase inhibitors (imatinib and sorafenib) reported a recurrence rate of 5.5% in a large series of 42 have demonstrated activity in the treatment of DTs because abdominal wall tumors . of the PDGFRs expressed in tumor stroma. Other prognostic factors for recurrence have been In a series published by Gounder et al., Sorafenib was identiﬁed, but they are still under investigation. In some administered at 400 mg oral daily. (ere was a 30% re- studies, tumors larger than 8 cm were more likely to recur duction of the tumor size in 92% of the patients . Penel (p � 0.021) . Crago et al. discovered that tumors larger et al. evaluated the eﬃcacy of Imatinib in patients with than 10 cm were more likely to relapse . Age at diagnosis unresectable and progressive symptomatic DTs. (is treat- was a signiﬁcant factor for recurrence in many studies. ment showed that 83% of patients had stable disease; 3% had Crago et al. found more recurrence in patients younger than complete response rate and 9% had partial response rate 26 years . In our series, tumor size and age were not . factors for local relapse. Due to the unpredictable behavior of DTs, such as long (ere are multiple protocols to treat DTs: simple sur- extended periods of stable disease and even rapid pro- veillance, surgery, chemotherapy radiation therapy, tyrosine gression, treatment must be individualized to improve the kinase inhibitors, hormonal treatment, and nonsteroidal eﬃciency of local tumor control and protect the quality of anti-inﬂammatory drugs. life. Consequently, the application of a multidisciplinary Some series compared a simple surveillance protocol assessment along with multimodality treatment shapes the versus an active treatment and showed that the 5 years foundation of care for the related patients. progression-free survival is almost the same (49.9% versus 58.6%) . 5. Conclusions In other series, a spontaneous regression was observed in 20% of cases . DTs are aggressive mesenchymal tumors with a tendency for (is result was in accordance with the wait-and-see local recurrence. Abdominal wall tumors have less risk of method. However, when patients are symptomatic and recurrence. Macroscopic margin involvement was the only experiencing rapid or life-threatening progression and the prognostic factor that aﬀects disease-free-survival. A mul- tumor is resectable with negative margins, surgery seems to tidisciplinary approach is mandatory. Treatment recom- be the best option . But, mutilating surgical resection mendations including surgery, radiation therapy, and and function loss are the disadvantages of surgery. systemic therapy are all evolving. Systemic treatment options for aggressive ﬁbromatosis that are not amenable to surgery or radiation therapy Data Availability comprise nonsteroidal anti-inﬂammatory drugs (NSAIDs), antihormonal therapies, tyrosine kinase inhibitors (TKIs), (e data that support the ﬁndings of this study are available and chemotherapy. from the corresponding author upon reasonable request. 6 International Journal of Surgical Oncology International Journal of Surgery Case Reports, vol. 30, Conflicts of Interest pp. 122–125, 2017.  H. Krentel, G. Tchartchian, and R. L. De Wilde, “Desmoid (e authors declare no conﬂicts of interest. tumor of the anterior abdominal wall in female patients: comparison with endometriosis,” Case Reports in Medicine, Authors’ Contributions vol. 2012, Article ID 725498, 4 pages, 2012.  S. L. Wong, “Diagnosis and management of desmoid tumors Yosr Zenzri was involved in the concepts, design, literature and ﬁbrosarcoma,” Journal of Surgical Oncology, vol. 97, no. 6, search, manuscript preparation, and statistical analysis; pp. 554–558, 2008. Yosra Yahyaoui was the guarantor and was responsible for  A. E. M. Righetti, C. Jacomini, R. S. Parra, A. L. N. R. d. Almeida, concepts, design, literature search, and manuscript prepa- J. J. R. Rocha, and O. Fe´res, “Familial adenomatous polyposis and ration; Lamia Charﬁ was involved in the deﬁnition of in- desmoid tumors,” Clinics, vol. 66, no. 10, pp. 1839–1842, 2011. tellectual content and design; Zahra Ghodhbani was  M. H. Nieuwenhuis, E. M. Mathus-Vliegen, C. G. Baeten et al., responsible for the literature search and manuscript prep- “Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients,” aration; Feryel Letaief and Mouna Ayadi prepared and British Journal of Cancer, vol. 104, no. 1, pp. 37–42, 2011. reviewed the manuscript; Amel Mezlini was responsible for  R. T. Zreik and K. J. Fritchie, “Morphologic spectrum of concepts and deﬁnition of intellectual content. desmoid-type ﬁbromatosis,” American Journal of Clinical Pathology, vol. 145, no. 3, pp. 332–340, 2016. References  J. T. Mullen, T. F. DeLaney, A. E. Rosenberg et al., “β-Catenin mutation status and outcomes in sporadic desmoid tumors,”  Y. Zhou, Z. Zhang, H. Fu, W. Qiu, L. Wang, and Y. He, /e Oncologist, vol. 18, no. 9, pp. 1043–1049, 2013. “Clinical management of pediatric aggressive ﬁbromatosis  E. Robanus-Maandag, C. Bosch, S. Amini-Nik et al., “Familial involving the mandible,” Pediatric Blood & Cancer, vol. 59, adenomatous polyposis-associated desmoids display signiﬁ- no. 4, pp. 648–651, 2012. cantly more genetic changes than sporadic desmoids,” PLoS  A. AlShammari, T. S. AlSumai, A. A. Alhudaib, H. Khalifa, One, vol. 6, no. 9, Article ID e24354, 2011. and M. Aburahmah, “Ultrasound guided sparing resection of  C. Liu, Y. Wu, S.-Y. Yu et al., “Increased β-catenin and c-myc locally recurrent abdominal wall desmoid tumor,” Journal of expression predict aggressive growth of non-functioning Surgical Case Reports, vol. 2019, no. 6, pp. 1–3, 2019. pituitary adenomas: an assessment using a tissue microarray-  V. P. de Camargo, M. L. Keohan, D. R. D’Adamo et al., based approach,” Molecular Medicine Reports, vol. 15, 2017. “Clinical outcomes of systemic therapy for patients with deep  T. L. Ng, A. M. Gown, T. S. Barry et al., “Nuclear beta-catenin ﬁbromatosis (desmoid tumor),” Cancer, vol. 116, no. 9, in mesenchymal tumors,” Modern Pathology, vol. 18, no. 1, pp. 2258–2265, 2010. pp. 68–74, 2005.  M. Santos, A. Rocha, V. Martins, and M. Santos, “Desmoid  D. Ganeshan, B. Amini, P. Nikolaidis, M. Assing, and tumours in familial adenomatous polyposis: review of 17 R. Vikram, “Current update on desmoid ﬁbromatosis,” patients from a Portuguese tertiary center,” Journal of Clinical Journal of Computer Assisted Tomography, vol. 43, no. 1, and Diagnostic Research, vol. 10, no. 10, pp. PC01–PC05, 2016. pp. 29–38, 2019.  A. L. Knudsen and S. Bulow, ¨ “Desmoid tumour in familial  S. X. Skapek, W. S. Ferguson, L. Granowetter et al., “Vin- adenomatous polyposis. A review of literature,” Familial blastine and methotrexate for desmoid ﬁbromatosis in chil- Cancer, vol. 1, no. 2, pp. 111–119, 2001. dren: results of a pediatric oncology group phase II trial,”  J. Y. Cho, S. Gupta, H. S. Cho et al., “Role of nonsteroidal anti- Journal of Clinical Oncology, vol. 25, no. 5, pp. 501–506, 2007. inﬂammatory drug in treatment of extra-abdominal desmoid  J. A. Jelinek, K. J. Stelzer, E. Conrad et al., “(e eﬃcacy of tumors,” Clinics in Orthopedic Surgery, vol. 10, no. 2, radiotherapy as postoperative treatment for desmoid tumors,” pp. 225–233, 2018. ∗ ∗ International Journal of Radiation Oncology Biology Physics,  B. Kasper, C. Baumgarten, J. Garcia et al., “An update on the vol. 50, no. 1, pp. 121–125, 2001. management of sporadic desmoid-type ﬁbromatosis: a Eu-  M. A. Spear, L. C. Jennings, H. J. Mankin et al., “Individu- ropean consensus initiative between sarcoma PAtients alizing management of aggressive ﬁbromatoses,” Interna- EuroNet (SPAEN) and European organization for research ∗ ∗ tional Journal of Radiation Oncology Biology Physics, vol. 40, and treatment of cancer (EORTC)/Soft tissue and bone no. 3, pp. 637–645, 1998. sarcoma group (STBSG),” Annals of Oncology, vol. 28, no. 10,  N. B. Merchant, J. J. Lewis, J. M. Woodruﬀ, D. H. Y. Leung, pp. 2399–2408, 2017. and M. F. Brennan, “Extremity and trunk desmoid tumors,”  S. Salas, A. Dufresne, B. Bui et al., “Prognostic factors Cancer, vol. 86, no. 10, pp. 2045–2052, 1999. inﬂuencing progression-free survival determined from a se-  R. J. Sutton and J. M. (omas, “Desmoid tumours of the ries of sporadic desmoid tumors: a wait-and-see policy anterior abdominal wall,” European Journal of Surgical On- according to tumor presentation,” Journal of Clinical On- cology (EJSO), vol. 25, no. 4, pp. 398–400, 1999. cology, vol. 29, no. 26, pp. 3553–3558, 2011.  Y. F. Shao, H. T. Yu, J. Q. Hu, and X. H. Yuan, “Abdominal  A. P. Molloy, B. Hutchinson, and G. C. O’Toole, “Extra-ab- wall desmoid tumor--analysis of 42 patients [Abstract],” dominal desmoid tumours: a review of the literature,” Sar- Zhonghua Zhong Liu Za Zhi, vol. 10, no. 1, pp. 63-64, 1988. coma, vol. 2012, Article ID 578052, 9 pages, 2012.  X. D. He, Y. B. Zhang, L. Wang et al., “Prognostic factors for  A. M. Crago, B. Denton, S. Salas et al., “A prognostic no- mogram for prediction of recurrence in desmoid ﬁbroma- the recurrence of sporadic desmoid-type ﬁbromatosis after tosis,” Annals of Surgery, vol. 258, no. 2, pp. 347–353, 2013. macroscopically complete resection: analysis of 114 patients at  S. Xuereb, R. Xuereb, C. Buhagiar, J. Gauci, and C. Magri, “A a single institution,” European Journal of Surgical Oncology case report of desmoid tumour-a forgotten aspect of FAP,” (EJSO), vol. 41, no. 8, pp. 1013–1019, 2015. International Journal of Surgical Oncology 7  B. Kasper, P. Strobel, ¨ and P. Hohenberger, “Desmoid tumors: clinical features and treatment options for advanced disease,” /e Oncologist, vol. 16, no. 5, pp. 682–693, 2011.  B. Alman, S. Attia, C. Baumgarten et al., “(e management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients,” European Journal of Cancer, vol. 127, pp. 96–107, 2020.  A. Hansmann, C. Adolph, T. Vogel, A. Unger, and G. Moeslein, “High-dose tamoxifen and sulindac as ﬁrst-line treatment for desmoid tumors,” Cancer, vol. 100, no. 3, pp. 612–620, 2004.  J. Janinis, M. Patriki, L. Vini, G. Aravantinos, and J. S. Whelan, “(e pharmacological treatment of aggressive ﬁbromatosis: a systematic review,” Annals of Oncology, vol. 14, no. 2, pp. 181–190, 2003.  D. Garbay, A. Le Cesne, N. Penel et al., “Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG),” Annals of Oncology, vol. 23, no. 1, pp. 182–186, 2012.  A. Constantinidou, R. L. Jones, M. Scurr, O. Al-Muderis, and I. Judson, “Pegylated liposomal doxorubicin, an eﬀective, well-tolerated treatment for refractory aggressive ﬁbroma- tosis,” European Journal of Cancer, vol. 45, no. 17, pp. 2930–2934, 2009.  M. M. Gounder, R. A. Lefkowitz, M. L. Keohan et al., “Activity of Sorafenib against desmoid tumor/deep ﬁbromatosis,” Clinical Cancer Research, vol. 17, no. 12, pp. 4082–4090, 2011.  N. Penel, A. Le Cesne, B. N. Bui et al., “Imatinib for pro- gressive and recurrent aggressive ﬁbromatosis (desmoid tu- mors): an FNCLCC/French Sarcoma Group phase II trial with a long-term follow-up,” Annals of Oncology, vol. 22, no. 2, pp. 452–457, 2011.
International Journal of Surgical Oncology – Hindawi Publishing Corporation
Published: Jul 18, 2020
Access the full text.
Sign up today, get DeepDyve free for 14 days.